NMOSD诊断和治疗

NMOSD诊断和治疗,主要内容,NMOSD定义和历史进展最新诊断标准MRI表现特点鉴别诊断病变主要治疗,TheDefinitionandProgressionofNMOSD,Neuromyelitisoptica(NMO)isaninflammatoryCNSsyndromedistinctfrommultiplesclerosis(MS)thatisassociatedwithserumaquaporin-4immunoglobulinGantibodies(AQP4-IgG).WithdeeperunderstandingofNMO,thediagnosticcriteriaofNMOalsoevolvedfromtheversionin1999,throughtherevisedonein2006,andfinallytothefirstinternationalconsensuscriteriain2015.ThenewcriteriahaveadoptedthebroadertermofNMOSDtoincludepatientswithlimitedmanifestations.Moreover,theyhavestratifiedNMOSDintotwotypes:NMOSDwithAQP4-IgG(NMOSD-AQP4);andNMOSDwithoutAQP4-IgGorwithunknownAQP4-IgGstatus.,NMOSDdiagnosticcriteriaforadultpatients,DiagnosticcriteriaforNMOSDwithAQP4-IgG1.Atleast1coreclinicalcharacteristic2.PositivetestforAQP4-IgGusingbestavailabledetectionmethod(cell-basedassaystronglyrecommended)3.ExclusionofalternativediagnosesDiagnosticcriteriaforNMOSDwithoutAQP4-IgGorNMOSDwithunknownAQP4-IgGstatus1.Atleast2coreclinicalcharacteristicsoccurringasaresultofoneormoreclinicalattacksandmeetingallofthefollowingrequirements:a.Atleast1coreclinicalcharacteristicmustbeopticneuritis,acutemyelitiswithLETM,orareapostremasyndromeb.Disseminationinspace(2ormoredifferentcoreclinicalcharacteristics)c.FulfillmentofadditionalMRIrequirements,asapplicable2.NegativetestsforAQP4-IgGusingbestavailabledetectionmethod,ortestingunavailable3.Exclusionofalternativediagnoses,Coreclinicalcharacteristics1.Opticneuritis2.Acutemyelitis3.Areapostremasyndrome:episodeofotherwiseunexplainedhiccupsornauseaandvomiting4.Acutebrainstemsyndrome5.SymptomaticnarcolepsyoracutediencephalicclinicalsyndromewithNMOSD-typicaldiencephalicMRIlesions6.SymptomaticcerebralsyndromewithNMOSD-typicalbrainlesions,AdditionalMRIrequirementsforNMOSDwithoutAQP4-IgGandNMOSDwithunknownAQP4-IgGstatus1.Acuteopticneuritis:requiresbrainMRIshowing(a)normalfindingsoronlynonspecificwhitematterlesions,OR(b)opticnerveMRIwithT2-hyperintenselesionorT1-weightedgadoliniumenhancinglesionextendingover.1/2opticnervelengthorinvolvingopticchiasm2.Acutemyelitis:requiresassociatedintramedullaryMRIlesionextendingover3contiguoussegments(LETM)OR3contiguoussegmentsoffocalspinalcordatrophyinpatientswithhistorycompatiblewithacutemyelitis3.Areapostremasyndrome:requiresassociateddorsalmedulla/areapostremalesions4.Acutebrainstemsyndrome:requiresassociatedperiependymalbrainstemlesions,SpinalcordMRI,acuteLETMlesionassociatedwithacuteTMIncreasedsignalonsagittalT2-weighted(standardT2-weighted,protondensity,orSTIRsequences)extendingover3ormorecompletevertebralsegmentsCentralcordpredominance(morethan70%ofthelesionresidingwithinthecentralgraymatter)GadoliniumenhancementofthelesiononT1-weightedsequences(nospecificdistributionorpatternofenhancementisrequired)OthercharacteristicfeaturesthatmaybedetectedRostralextensionofthelesionintothebrainstem(figure1,DandE)Cordexpansion/swellingDecreasedsignalonT1-weightedsequencescorrespondingtoregionofincreasedT2-weightedsignal,MRIPresentationsofNMOSD,SpinalcordMRI,chronicLongitudinallyextensivecordatrophy(sharplydemarcatedatrophyextendingovermorethan3complete,contiguousvertebralsegmentsandcaudaltoaparticularsegmentofthespinalcord),withorwithoutfocalordiffuseT2signalchangeinvolvingtheatrophicsegmentOpticnerveMRIUnilateralorbilateralincreasedT2signalorT1gadoliniumenhancementwithinopticnerveoropticchiasm(figure1,IK);relativelylonglesions(e.g.,thoseextendingmorethanhalfthedistancefromorbittochiasm)andthoseinvolvingtheposterioraspectsoftheopticnervesorthechiasmareassociatedwithNMO,CerebralMRI:NMOSD-typicalbrainlesionpatterns(increasedsignalonT2-weightedMRIsequencesunlessotherwisenoted)Lesionsinvolvingthedorsalmedulla(especiallytheareapostrema),eithersmallandlocalized,oftenbilateral,orcontiguouswithanuppercervicalspinalcordlesionPeriependymalsurfacesofthefourthventricleinthebrainstem/cerebellumLesionsinvolvingthehypothalamus,thalamus,orperiependymalsurfacesofthethirdventricleLarge,confluent,unilateral,orbilateralsubcorticalordeepwhitematterlesionsLong(1/2ofthelengthofthecorpuscallosumorgreater),diffuse,heterogeneous,oredematouscorpuscallosumlesionsLongcorticospinaltractlesions,unilateralorbilateral,contiguouslyinvolvinginternalcapsuleandcerebralpeduncleExtensiveperiependymalbrainlesions,oftenwithgadoliniumenhancement,DifferentialDiagnoses,Diverseneurologicaldiseasesincludinginflammatory,infectious,malignant,vascular,andhereditaryetiologiescanresemblethephenotypesofNMOSD.Nevertheless,astheseNMOSDmimicsaredistinctfromNMOSDintreatmentaswellaspathophysiology,earlydifferentialdiagnosisandappropriateindividualizedtreatmentwillimprovetheoutcomeofsuchpatients.,MultiplesclerosisAcutedisseminatedencephalomyelitisIdiopathicacutetransversemyelitisIdiopathicopticneuritisInflammatorydiseasesassociatedwithantibodytomyelinoligodendrocyteglycoproteinSarcoidosisCNSinvolvementinpatientswithsystemicautoimmunediseaseSjogrenssyndrome干燥综合征Systemiclupuserythematous.CNSlymphomaNeuro-BehetdiseaseSpinalduralarteriovenousfistulaInfectionsSyphilisMiscellaneousinfectionsLeberhereditaryopticneuropathy,治疗,NMOSD治疗应该遵循在循证医学证据的基础上，结合患者的经济条件和意愿，进行早期、合理、治疗目前NMOSD的治疗推荐主要是基于一些小样本临床试验、回顾性研究、以及专家共识并借助其他自身免疫性疾病治疗经验而得出NMOSD的治疗分为急性期治疗、序贯治疗（免疫抑制治疗）、对症治疗和康复治疗,急性期治疗（与MS相同）大剂量MTP冲击血浆置换缓解期治疗（与MS不同）硫唑嘌呤利妥昔单抗麦考酚酸莫酯,NMOSD对症治疗,抑郁(depression)草酸艾司，西酞普兰、氟西汀